肾上腺区巨大肿瘤合并瘤栓的诊断与治疗

doi:10.19723/j.issn.1671-167X.2019.04.015

摘要/Abstract

摘要：

目的:总结临床上少见的肾上腺区巨大肿瘤合并静脉瘤栓的诊断和手术经验。方法:自2017年10月至2019年3月,共8例肾上腺区巨大肿瘤(>7cm)合并静脉瘤栓病例就诊于北京大学第三医院,包括男性5例、女性3例,平均年龄50.6岁(31~62岁),右侧6例、左侧2例。首发症状包括腹部不适、高血压、Cushing面容、月经异常;特殊既往史包括肝硬化、肺癌。所有病例行影像学和内分泌学检查,术前影像学已明确合并下腔静脉瘤栓5例、左肾静脉瘤栓1例;术中发现合并肾上腺中央静脉瘤栓2例。4例右侧合并下腔静脉瘤栓且瘤栓上极较高病例实施开放肿瘤切除及瘤栓切除术,3例行腹腔镜手术(右侧2例、左侧1例),1例未行手术治疗。结果:平均肿瘤大小8.9 cm(7~11 cm),平均手术时间319 min(120~510 min),平均出血量629 mL(50~1 200 mL),术中输血2例,伤口感染1例。肿瘤类型:嗜铬细胞瘤1例、肾上腺皮质癌3例、肾上腺转移瘤2例、肾上腺区平滑肌肉瘤2例。术中发现嗜铬细胞瘤瘤栓中混合机化血栓;肾上腺皮质癌层次欠清,包膜极易破;肾上腺转移瘤血供丰富;肾上腺区平滑肌肉瘤均侵犯下腔静脉形成瘤栓,血供不丰富。平均随访时间8.4个月(1~15个月),1例肾上腺皮质癌术后4个月发生肝转移,于1年后死亡。结论:合并瘤栓的肾上腺区巨大肿瘤种类多,术前诊断有一定难度,需全面评估和完善术前准备;其手术难度大,方式多样,开放手术以及腹腔镜手术均可行。

关键词: 肾上腺肿瘤, 瘤栓, 诊断, 外科手术, 病理学, 临床

Abstract:

Objective: To summarize the experience of diagnosis and operation related to massive adrenal area tumor with venous tumor thrombus in clinic.Methods: From October 2017 to March 2019, a total of 8 cases of massive adrenal area tumor (>7 cm) with venous tumor thrombus were admitted at Peking University Third Hospital including 5 males and 3 females with mean age 50.6 years (31-62 years). There were 6 cases on the right side and 2 cases on the left side. The first symptoms included abdominal discomfort, hypertension, Cushing syndrome and abnormal menstruation; special past history included cirrhosis and lung cancer. Computed tomography (CT) and routine endocrine hormone tests were examined. Preoperative imaging confirmed 5 cases masses with tumor thrombus in inferior vena cava (IVC) and 1 case with tumor thrombus in left renal vein. Two cases presented with tumor thrombus in central adrenal vein were found intraoperatively. Open adrenalectomy and thrombectomy were performed in 4 cases of right side complicated with high tumor thrombus of inferior vena cava. Laparoscopic adrenalectomy and thrombectomy were performed in 3 cases, including 2 cases on the right and 1 case on the left. The case with tumor thrombus in left renal vein gave up operation. The patients were followed up with outpatient and telephone.Results: The mean size of the tumor was 8.9 cm (7-11 cm), the mean operative time was 319 min (120-510 min), while the estimated blood loss was 629 mL (50-1 200 mL). Intraoperative blood transfusion was required in 2 cases and 1 case encountered wound infection. The pathological types included pheochromocytoma, adrenocortical carcinoma, adrenal metastases of haptic carcinoma, and leiomyosarcoma. The case with left lung carcinoma who underwent left pneumonectomy one month before was highly suspected adrenal metastases of lung carcinoma. Tumor thrombus of pheochromocytoma was combined with blood thrombus. Capsule of adrenocortical carcinoma was fragile in adrenocortical carcinoma. Abundant blood supply existed in adrenal metastases. The leiomyosarcoma had unabundant blood supply and invaded IVC to form tumors thrombus. The mean follow-up time was 8.4 months (1-15 months). One case with adrenocortical carcinoma died after 1 year in the follow-up.Conclusion: We present the rare cases of different pathological types of massive adrenal tumors with tumor thrombus extending into the IVC extension or other vein. Preoperative diagnosis needs comprehensive evaluation and perfect preoperative preparation. Surgery is difficult and varied. Open approach as well as retroperitoneal laparoscopic approach is feasible.

Key words: Adrenal gland neoplasms, Tumor thrombus, Diagnosis, Surgical procedures,operative, Pathology,clinical

中图分类号:

R736.6

刘磊,王国良,马潞林,陆敏,刘承,侯小飞. 肾上腺区巨大肿瘤合并瘤栓的诊断与治疗[J]. 北京大学学报（医学版）, 2019, 51(4): 684-688.

Lei LIU,Guo-liang WANG,Lu-lin MA,Min LU,Cheng LIU,Xiao-fei HOU. Diagnosis and surgical treatment of massive adrenal area tumor with tumor thrombus[J]. Journal of Peking University(Health Sciences), 2019, 51(4): 684-688.

图/表 2

图1

表1

参考文献 13

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Items	Case 1	Case 2	Case 3	Case 4
Gender	Male	Female	Male	Male
Age/years	44	31	62	56
Symptom	Hypertension	Cushing syndrome and virilization	History of cirrhosis	History of lung cancer
Diagnosis	Pheochromocytoma	Adrenocortical carcinoma	Metastases of heptic carcinoma	Metastases of lung carcinoma
Hormone test	Hypercatecholemia	Hypercortisolism	Normal	Normal
Side	Right	Right	Right	Left
Size/cm	9	11	8	9
Tumor thrombus	3 cm in IVC	4 cm in IVC	3 cm in IVC	3 cm in left renal vein
Approach	Open	Open	Open
Surgical duration/min	400	510	380
Blood loss/mL	400	1 000	750
Follow-up/months	15	Death after 12	12	Loss
Items	Case 5	Case 6	Case 7	Case 8
Gender	Female	Female	Male	Male
Age/years	57	42	58	55
Symptom	None	Cushing syndrome	None	None
Diagnosis	Leiomyosarcoma	Adrenocortical carcinoma	Adrenocortical carcinoma	Leiomyosarcoma
Hormone test	Normal	Hypercortisolism	Normal	Normal
Side	Right	Left	Right	Right
Size/cm	8	8	7	11
Tumor thrombus	3 cm in IVC invaded directly	1 cm in left principal adrenal vein	1 cm in principal adrenal vein	7 cm in IVC
Approach	Retroperitoneal laparoscopic	Retroperitoneal laparoscopic	Retroperitoneal laparoscopic	Open
Surgical duration/min	360	120	200	260
Blood loss/mL	1 200	50	200	800
Follow-up/months	7	7	5	1